Clinical features of neuromyelitis optica combined with or without connective tissue disease

Abstract

Objective To study the symptoms, clinical manifestations, laboratory tests, imaging, treatment and prognosis of neuromyelitis optica (NMOSD) with or without combined connective tissue disease (CTD). Methods The 226 NMOSD patients diagnosed at Ningxia Medical University General Hospital were divided into two groups according to the presence or absence of concurrent CTD. The clinical data and prognosis of NMOSD patients with combined CTD were analyzed and followed up. At the same time, NMOSD patients with combined CTD were compared with NMOSD patients without the presence of CTD in terms of laboratory tests, imaging tests, and the similarities and differences between the two groups were analyzed. Results CTD combined with NMOSD mostly occurred in females, and the most prevalent form of CTD is systemic lupus erythematosus (SLE) or primary Sjögren's syndrome (pSS). It has a variety of symptoms, the majority of which are visual loss or limb weakness and numbness, and the majority of patients are positive for AQP4-IgG. The symptoms resolve significantly after sequential hormonal shock therapy in the acute phase, but the relapse rate is high. Meanwhile, it was revealed that when compared between NMOSD patients with combined CTD and NMOSD patients without combined CTD, there was a significant difference between the recurrent events (including recurrence rate and number of recurrences) and degree of remission (p<0.05). Conclusions The high incidence of CTD combined with NMOSD in women and the high recurrence rate suggest that adjuvant immunosuppression should be given to further reduce recurrence.